Cargando…
Granulomatosis with polyangiitis: a 17 year experience from a tertiary care hospital in Pakistan
OBJECTIVE: Granulomatosis with Polyangiitis (GPA) is an autoimmune, multi-system, small and medium vessel vasculitis with granulomatous inflammation. Aim of this study was to assess the clinical and radiological presentations of patients with GPA amongst the Pakistani population. It is a single cent...
Autores principales: | , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2018
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5956959/ https://www.ncbi.nlm.nih.gov/pubmed/29769093 http://dx.doi.org/10.1186/s13104-018-3434-2 |
_version_ | 1783323982578057216 |
---|---|
author | Irfan, Omar Khan, Haris Khan, Zarrar Ashraf, Alina Ahmed, Rimsha Khan, Javaid Ahmad Zubairi, Ali Bin Sarwar |
author_facet | Irfan, Omar Khan, Haris Khan, Zarrar Ashraf, Alina Ahmed, Rimsha Khan, Javaid Ahmad Zubairi, Ali Bin Sarwar |
author_sort | Irfan, Omar |
collection | PubMed |
description | OBJECTIVE: Granulomatosis with Polyangiitis (GPA) is an autoimmune, multi-system, small and medium vessel vasculitis with granulomatous inflammation. Aim of this study was to assess the clinical and radiological presentations of patients with GPA amongst the Pakistani population. It is a single centre retrospective single observation study. RESULTS: Study was conducted at the Aga Khan University Hospital, Karachi with records were reviewed from January 2000 to December 2017. Definitive diagnosis was made using a combination of serological anti-neutrophil cytoplasmic antibody (ANCA) testing along with the clinical and radiological presentation. A total of 51 patients met the diagnostic criteria in the time frame of the study. There were 23 males and 28 females with mean age of 44.0 ± 17.8 years on presentation. Arthritis was the most common symptom present in 41.2% of the cases followed by cough in 32.0%. Sixteen patients showed pulmonary infiltrates on chest X-ray. C-ANCA was positive in all of the patients compared with 21.6% p-ANCA positivity. A total of 13 biopsies were done. The median Birmingham Vasculitis Activity Score was 12. We report a 17.6% mortality rate with 5 deaths occurring due to respiratory failure. GPA is a diagnostic challenge leading to late diagnosis which can contribute to significant morbidity and mortality specially in the Third World. |
format | Online Article Text |
id | pubmed-5956959 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-59569592018-05-24 Granulomatosis with polyangiitis: a 17 year experience from a tertiary care hospital in Pakistan Irfan, Omar Khan, Haris Khan, Zarrar Ashraf, Alina Ahmed, Rimsha Khan, Javaid Ahmad Zubairi, Ali Bin Sarwar BMC Res Notes Research Note OBJECTIVE: Granulomatosis with Polyangiitis (GPA) is an autoimmune, multi-system, small and medium vessel vasculitis with granulomatous inflammation. Aim of this study was to assess the clinical and radiological presentations of patients with GPA amongst the Pakistani population. It is a single centre retrospective single observation study. RESULTS: Study was conducted at the Aga Khan University Hospital, Karachi with records were reviewed from January 2000 to December 2017. Definitive diagnosis was made using a combination of serological anti-neutrophil cytoplasmic antibody (ANCA) testing along with the clinical and radiological presentation. A total of 51 patients met the diagnostic criteria in the time frame of the study. There were 23 males and 28 females with mean age of 44.0 ± 17.8 years on presentation. Arthritis was the most common symptom present in 41.2% of the cases followed by cough in 32.0%. Sixteen patients showed pulmonary infiltrates on chest X-ray. C-ANCA was positive in all of the patients compared with 21.6% p-ANCA positivity. A total of 13 biopsies were done. The median Birmingham Vasculitis Activity Score was 12. We report a 17.6% mortality rate with 5 deaths occurring due to respiratory failure. GPA is a diagnostic challenge leading to late diagnosis which can contribute to significant morbidity and mortality specially in the Third World. BioMed Central 2018-05-16 /pmc/articles/PMC5956959/ /pubmed/29769093 http://dx.doi.org/10.1186/s13104-018-3434-2 Text en © The Author(s) 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Research Note Irfan, Omar Khan, Haris Khan, Zarrar Ashraf, Alina Ahmed, Rimsha Khan, Javaid Ahmad Zubairi, Ali Bin Sarwar Granulomatosis with polyangiitis: a 17 year experience from a tertiary care hospital in Pakistan |
title | Granulomatosis with polyangiitis: a 17 year experience from a tertiary care hospital in Pakistan |
title_full | Granulomatosis with polyangiitis: a 17 year experience from a tertiary care hospital in Pakistan |
title_fullStr | Granulomatosis with polyangiitis: a 17 year experience from a tertiary care hospital in Pakistan |
title_full_unstemmed | Granulomatosis with polyangiitis: a 17 year experience from a tertiary care hospital in Pakistan |
title_short | Granulomatosis with polyangiitis: a 17 year experience from a tertiary care hospital in Pakistan |
title_sort | granulomatosis with polyangiitis: a 17 year experience from a tertiary care hospital in pakistan |
topic | Research Note |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5956959/ https://www.ncbi.nlm.nih.gov/pubmed/29769093 http://dx.doi.org/10.1186/s13104-018-3434-2 |
work_keys_str_mv | AT irfanomar granulomatosiswithpolyangiitisa17yearexperiencefromatertiarycarehospitalinpakistan AT khanharis granulomatosiswithpolyangiitisa17yearexperiencefromatertiarycarehospitalinpakistan AT khanzarrar granulomatosiswithpolyangiitisa17yearexperiencefromatertiarycarehospitalinpakistan AT ashrafalina granulomatosiswithpolyangiitisa17yearexperiencefromatertiarycarehospitalinpakistan AT ahmedrimsha granulomatosiswithpolyangiitisa17yearexperiencefromatertiarycarehospitalinpakistan AT khanjavaidahmad granulomatosiswithpolyangiitisa17yearexperiencefromatertiarycarehospitalinpakistan AT zubairialibinsarwar granulomatosiswithpolyangiitisa17yearexperiencefromatertiarycarehospitalinpakistan |